Factor XIII Deficiency and Intracranial Bleed: Surgical Management and Prophylaxis with Cryoprecipitate

Abstract

Factor XIII (FXIII) deficiency is a rare bleeding disorder with affected patients having high propensity for intracranial hemorrhage. A 12-year-old girl presented with severe headache, limb weakness, and rapidly worsening sensorium over 4 days. Magnetic resonance imaging of the brain and computed tomography (CT) of the head showed intraparenchymal bleed. Patient had normal coagulation profile and abnormal FXIII level. The perioperative management included cryoprecipitate transfusion to bring the FXIII value to 74%. She underwent craniotomy and evacuation of the hematoma. Postoperatively, she received prophylaxis against rebleed with cryoprecipitate. In the absence of FXIII concentrate, correction of FXIII deficiency is possible with cryoprecipitate in emergent situations.

Keywords

brain - craniotomy - cryoprecipitate - factor XIII deficiency - intraparenchymal bleed

Publication History

Received: 27 January 2021

Accepted: 26 March 2021

Article published online:
19 June 2021

• References

• 1 Perez DL, Diamond EL, Castro CM. et al. Factor XIII deficiency related recurrent spontaneous intracerebral hemorrhage: a case and literature review. Clin Neurol Neurosurg 2011; 113 (02) 142-145
• 2 Nijenhuis AV, van Bergeijk L, Huijgens PC, Zweegman S. Acquired factor XIII deficiency due to an inhibitor: a case report and review of the literature. Haematologica 2004; 89 (05) ECR14
• 3 Alavi SER, Jalalvand M, Assadollahi V, Tabibian S, Dorgalaleh A. Intracranial hemorrhage: a devastating outcome of congenital bleeding disorders-prevalence, diagnosis, and management, with a special focus on congenital factor XIII deficiency. Semin Thromb Hemost 2018; 44 (03) 267-275
• 4 Aydin Ozturk P, Sanri O, Yilmaz A, Arpa A, Ozturk U, Ceviz A. Choroid plexus papilloma and factor XIII deficiency: case report. Pediatr Neurosurg 2018; 53 (06) 413-415
• 5 Solomon C, Korte W, Fries D. et al. Safety of factor XIII concentrate: analysis of more than 20 years of pharmacovigilance data. Transfus Med Hemother 2016; 43 (05) 365-373
• 6 Hsieh L, Nugent D. Factor XIII deficiency. Haemophilia 2008; 14 (06) 1190-1200
• 7 Robbins KC. A study of the conversion of the fibrinogen to fibrin. Am J Physiol 1944; 142: 581-588
• 8 Poornima P, Shashibhushan KK, Bharath KP, Subba Reddy VV. Factor XIII deficiency: report of two cases. J Clin Pediatr Dent 2010; 35 (01) 101-104
• 9 Shanbhag S, Ghosh K, Shetty S. Genetic basis of severe factor XIII deficiency in a large cohort of Indian patients: Identification of fourteen novel mutations. Blood Cells Mol Dis 2016; 57: 81-84
• 10 Droubatchevskaia N, Wong MP, Chipperfield KM, Wadsworth LD, Ferguson DJ. Guidelines for cryoprecipitate transfusion. BCMJ 2007; 49 (08) 441-445
• 11 Odame JE, Chan AK, Wu JK, Breakey VR. Factor XIII deficiency management: a review of the literature. Blood Coagul Fibrinolysis 2014; 25 (03) 199-205
• 12 Adelmann D, Klaus DA, Illievich UM. et al. Fibrinogen but not factor XIII deficiency is associated with bleeding after craniotomy. Br J Anaesth 2014; 113 (04) 628-633
• 13 Gerlach R, Raabe A, Zimmermann M, Siegemund A, Seifert V. Factor XIII deficiency and postoperative hemorrhage after neurosurgical procedures. Surg Neurol 2000; 54 (03) 260-264 , discussion 264–265
• 14 Nielsen VG, Gurley Jr WQ, Burch TM. The impact of factor XIII on coagulation kinetics and clot strength determined by thrombelastography. Anesth Analg 2004; 99 (01) 120-123
• 15 Caudill JS, Nichols WL, Plumhoff EA. et al. Comparison of coagulation factor XIII content and concentration in cryoprecipitate and fresh-frozen plasma. Transfusion 2009; 49 (04) 765-770